| Acute lymphoid leukemia associated with Maffucci's syndrome. | |
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MedLine Citation:
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PMID: 8214367 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE: Maffucci's syndrome is a nonhereditary congenital disorder associated with multiple enchondromas, soft tissue hemangiomas, or lymphangiomas. It carries an associated high risk of the development of malignant neoplasms, particularly sarcomatous transformation of an enchondroma, as well as other malignant mesodermal and nonmesodermal neoplasms. Hematopoietic malignancies arising in Maffucci's syndrome are exceedingly rare. We report the case of a 14-year-old girl with Maffucci's syndrome who developed acute lymphoid leukemia. PATIENTS AND METHODS: The patient presented at 18 months of age with enchondromatosis. Maffucci's syndrome was established at 10 years of age after the appearance of multiple hemangiomas. RESULTS: At 14 years of age the patient developed fatigue, frequent nosebleeds, easy bruising, and weight loss, with circulating blasts in the peripheral blood. Bone marrow examination showed replacement of marrow spaces with leukemic blasts. Immunohistochemical and flow cytometric findings were consistent with a diagnosis of acute lymphoblastic leukemia with myeloid antigen expression. CONCLUSIONS: The occurrence of acute leukemia in a patient with Maffucci's syndrome may represent predisposition to yet another malignancy and reflect further expression of a generalized mesodermal dysplasia in these patients. It also emphasizes the need for aggressive surveillance in patients with Maffucci's syndrome. |
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Authors:
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J T Rector; C L Gray; R W Sharpe; F W Hall; W Thomas; W Jones |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: The American journal of pediatric hematology/oncology Volume: 15 ISSN: 0192-8562 ISO Abbreviation: Am J Pediatr Hematol Oncol Publication Date: 1993 Nov |
Date Detail:
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Created Date: 1993-11-19 Completed Date: 1993-11-19 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7908071 Medline TA: Am J Pediatr Hematol Oncol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 427-9 Citation Subset: IM |
Affiliation:
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Department of Laboratory Medicine, Naval Hospital, San Diego, California 92134-5000. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Antigens, CD / blood Bone Marrow / pathology Enchondromatosis / complications*, pathology, radiography Female Hemangioma / complications, pathology Humans Lymphocytes / immunology Precursor Cell Lymphoblastic Leukemia-Lymphoma / blood, complications*, immunology, pathology Skin Neoplasms / complications, pathology |
| Chemical | |
Reg. No./Substance:
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0/Antigens, CD |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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